Essential mixed cryoglobulinemia
Evidence: Immune-mediated
Names
Essential mixed cryoglobulinemia
Cryoglobulinemic Vasculitis
Autoimmune cryoglobulinemia
Description
Mixed cryoglobulinemia is marked by the presence of abnormal antibodies (cryoglobulins) that clump together and become solid or gel-like at temperatures below normal body temperature (98.6 F). The exact temperature at which this occurs may vary from one person to another.
When these proteins clump together in the bloodstream, they block the flow of blood to joints, muscles, and organs. Eventually, damage or inflammation of affected blood vessels (vasculitis) and surrounding tissue can develop.
Cryoglobulinemia is classified as follows:
Type I cryoglobulinemia (simple cryoglobulinemia), involves monoclonal immunoglobulin, usually immunoglobulin M (IgM) or, less frequently, immunoglobulin G (IgG), immunoglobulin A (IgA), or light chains.
Types II and III cryoglobulinemia (mixed cryoglobulinemia) include rheumatoid factors (RFs) that are usually IgM and rarely IgG or IgA. Type II involves monoclonal rheumatoid factors; type III involves polyclonal rheumatoid factors. Types II and III cryoglobulinemia represent 80% of all cryoglobulins.
Patient Groups
Bloggers
None found.
Prevalence
No data found. Autoimmune forms of essential mixed cryoglobulinemia represent only 15% of those with the disease, which is usually associated with hepatitis C infection [Mascia 2007].
Typical Age of onset
Age 40 to 60
Symptoms
Cryoglobulinemia does not have specific symptoms. Symptoms depend on which organs or tissues are damaged by the lack of circulation caused by the clumping of protiens.
The most common observable symptoms are caused when the skin, or tissue just below the skin, are affected by lack of circulation. In these cases the following symptoms may be observed:
· purplish discoloration of the skin (purpura)
· small lesions the size of pinpricks (petechaie)
· larger lesions or bruising (ecchymoses)
· open sores (ulceration)
· hives (urticarial)
· tissue damage (necrosis) on the extremities (e.g. fingers and toes)
The lower extremities (legs, feet) are most often affected, and symptoms are most commonly observed after an individual has been standing or sitting for long periods of time (orthostatic purpura).
